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International Journal of Hematology

, Volume 75, Issue 4, pp 382–384 | Cite as

The Effect of Thrombopoietin on Erythroid Progenitors in Diamond-Blackfan Anemia

  • Hirohide Kawasaki
  • Takahide Nakano
  • Urara Kohdera
  • Yohnosuke Kobayashi
Review Article

Abstract

Diamond-Blackfan anemia (DBA) is a rare congenital red blood cell aplasia. Usually, erythropoiesis in vitro is defective, and decreased numbers of erythroid progenitors are found in colony assays performed with bone marrow cells. Recently, some investigators showed that thrombopoietin (TPO) also works on common multipotent progenitor cells and stimulates erythropoiesis. We examined the effect of TPO together with erythropoietin (EPO), stem cell factor (SCF), and/or interleukin-3 (IL-3) on erythroid burst-forming units (BFU-E) of bone marrow nonadherent mononuclear cells from 2 patients with DBA using a serum-free culture system.Very few BFU-E appeared in cultures containing EPO alone.Adding IL-3, SCF, or both to cultures containing EPO induced an increase in the number of BFU-E. When TPO was added to cultures containing EPO and IL-3, SCF, or both, the number of BFU-E further increased. Especially in patient 2, BFU-E formation was grossly equal to that in normal controls. We conclude that TPO enhances bone marrow erythropoiesis in patients with DBA in the presence of EPO and SCF and/or IL-3. The data raise the possibility of the combination of TPO and SCF as a therapeutic agent in DBA.

Key words

Congenital pure red cell aplasia Diamond-Blackfan anemia Erythroid progenitor cells Erythropoiesis Thrombopoietin 

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Copyright information

© The Japanese Society of Hematology 2002

Authors and Affiliations

  • Hirohide Kawasaki
    • 1
  • Takahide Nakano
    • 1
  • Urara Kohdera
    • 1
  • Yohnosuke Kobayashi
    • 1
  1. 1.Department of PediatricsKansai Medical UniversityOsakaJapan

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